Causes

The cause of total anomalous pulmonary venous return (TAPVR) is unknown.

In normal circulation, blood is sent from the right ventricle to pick up oxygen in the lungs. It then returns through the pulmonary (lung) veins to the left side of the heart, which sends blood out through the aorta and around the body.

In TAPVR, oxygen-rich blood returns from the lungs to the right atrium or to a vein flowing into the right atrium, instead of the left side of heart. In other words, blood simply circles to and from the lungs and never gets out to the body.

For the infant to live, a large atrial septal defect (ASD) or patent foramen ovale (passage between the left and right atria) must exist to allow oxygenated blood to flow to the left side of the heart and the rest of the body.

How severe this condition is depends on whether the pulmonary veins are blocked. The pulmonary veins may run into the abdomen, passing through a muscle (diaphragm). This muscle squeezes the veins and narrows them, causing the blood to back up into the lungs. This type of TAPVR causes symptoms early in life and can be deadly very quickly if it is not found and corrected with surgery.

Symptoms

The infant may appear very sick and may have the following symptoms:

• Bluish color of the skin (cyanosis)
• Frequent respiratory infections
• Lethargy
• Poor feeding
• Poor growth
• Rapid breathing      

Note: Sometimes, no symptoms may be present in infancy or early childhood.

Exams and Tests

• Cardiac catheterization can confirm the diagnosis by showing that the blood vessels are abnormally attached
• ECG shows enlargement of the ventricles (ventricular hypertrophy)
• Echocardiogram may show that the pulmonary vessels are attached
• MRI of the heart can show the connections between the pulmonary vessels
• X-ray of the chest shows a normal to small heart with fluid in the lungs

Treatment

Surgery to repair the problem is needed as soon as possible. In surgery, the pulmonary veins are connected to the left atrium and the defect between the right and left atrium is closed.

Outlook (Prognosis)

If this condition is not treated, the heart will get larger, leading to heart failure. Repairing the defect early provides excellent results if there is no blockage of the pulmonary veins at the new connection into the heart.

Possible Complications

• Breathing difficulties
• Heart failure
• Irregular, fast heart rhythms (arrhythmias)
• Lung infections
• Pulmonary hypertension

When to Contact a Medical Professional

This condition may be apparent at the time of birth. However, symptoms may not be present until later.

Call your health care provider if you notice symptoms of TAPVR -- prompt attention is required.

Prevention

There is no known way to prevent TAPVR.

Alternative Names

TAPVR; Total veins

References

Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 9th ed. Philadelphia, Pa:Saunders Elsevier; 2011:chap 65.

Updated: 4/1/2012

Reviewed by: Kurt R. Schumacher, MD, Pediatric Cardiology, University of Michigan Congenital Heart Center, Ann Arbor, MI. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.